Skip to main content
Log in

A case of antiphospholipid syndrome presenting with pulmonary truncus and main pulmonary artery thrombosis

  • Case Report
  • Published:
Rheumatology International Aims and scope Submit manuscript

Abstract

In patients with antiphospholipid syndrome (APS), thromboembolism and pulmonary hypertension are the most common pulmonary manifestations. Thrombotic obstruction at the level of the main and/or proximal pulmonary arteries is rare. We report a 40-year-old woman without any history of previous arterial and/or venous thrombosis who presented with severe dyspnea and was found to have pulmonary hypertension and positivity for anticardiolipin antibodies. Computed tomography revealed pulmonary truncus thrombosis extending to both right and left pulmonary arteries. The patient and her family refused surgical treatment. She had a prolonged hospital course, was unresponsive to thrombolytic, anticoagulant, antiplatelet, and immunosuppressive treatments, and died of right ventricle and respiratory failure 5 weeks later. This is the first reported case with thrombosis of pulmonary truncus and main pulmonary arteries concurrent with APS.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Greaves M, Cohen H, MacHin SJ, Mackie I (2000) Guidelines on the investigation and management of the antiphospholipid syndrome. Br J Haematol 109:704–715

    Article  CAS  PubMed  Google Scholar 

  2. Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT, Jacobsen S, Lakos G, Tincani A, Kontopoulou-Griva I et al, European Phospholipid Project Group (2002) Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 46:1019–1027

    Article  PubMed  Google Scholar 

  3. Asherson RA, Cervera R (1995) Review: antiphospholipid antibodies and the lung. J Rheumatol 22:62–66

    CAS  PubMed  Google Scholar 

  4. Piette JC, Hunt BJ (2000) Pulmonary hypertension and antiphospholipid antibodies. In: Khamashta MA (ed) Hughes syndrome. Antiphospholipid syndrome. Springer, London, pp 96–104

  5. Vianna JL, Khamashta MA, Ordi-Ros J, Font J, Cervera R, Lopez-Soto A, Tolosa C, Franz J, Selva A, Ingelmo M et al (1994) Comparison of the primary and secondary antiphospholipid syndrome: a European multicenter study of 114 patients. Am J Med 96:3–9

    Article  CAS  PubMed  Google Scholar 

  6. Amigo MC, Khamashta MA, Hughes GR (1998) Antiphospholipid syndrome in SLE. Baillieres Clin Rheumatol 12:477–493

    CAS  PubMed  Google Scholar 

  7. Ando M, Takamoto S, Okita Y, Matsukawa R, Nakanishi N, Kyotani S, Satoh T (1998) Operation for chronic pulmonary thromboembolism accompanied by thrombophilia in 8 patients. Ann Thorac Surg 66:1919–1924

    Article  CAS  PubMed  Google Scholar 

  8. Luchi ME, Asherson RA, Lahita RG (1992) Primary idiopathic pulmonary hypertension complicated by pulmonary arterial thrombosis. Association with antiphospholipid antibodies. Arthritis Rheum 35:700–705

    CAS  PubMed  Google Scholar 

  9. Cucurull E, Ordi Ros J, Murtra M, Mellibovsky L, Orriols R, Vilardell M (1996) Pulmonary thromboendarterectomy in a patient with primary antiphospholipid syndrome. Med Clin (Barc) 106:498–500

    Google Scholar 

  10. Sandoval J, Amigo MC, Barragan R, Izaguirre R, Reyes PA, Martinez-Guerra ML, Palomar A, Gomez A, Garcia-Torres R (1996) Primary antiphospholipid syndrome presenting as chronic thromboembolic pulmonary hypertension. Treatment with thromboendarterectomy. J Rheumatol 23:772–775

    CAS  PubMed  Google Scholar 

  11. Nakagawa Y, Masuda M, Shiihara H, Tsuruta Y, Abe H, Miura M, Tanaka H (1992) Successful pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension associated with anticardiolipin antibodies: report of a case. Surg Today 22:548–552

    CAS  PubMed  Google Scholar 

  12. Nakajima T, Ando H, Ueno Y, Umadome H, Takahashi Y, Okano Y, Takamoto S (1997) Successful thromboendarterectomy for chronic pulmonary embolism in a patient with systemic lupus erythematosus and antiphospholipid syndrome. Jpn Circ J 61:958–964

    Article  CAS  PubMed  Google Scholar 

  13. Hartz RS, Byrne JG, Levitsky S, Park J, Rich S (1996) Predictors of mortality in pulmonary thromboendarterectomy. Ann Thorac Surg 62:1255–1259

    Article  CAS  PubMed  Google Scholar 

  14. Manson JJ, Isenberg DA (2003) Antiphospholipid syndrome. Int J Biochem Cell Biol 35:1015–1020

    Article  CAS  PubMed  Google Scholar 

  15. Asherson RA, Cervera R, Piette JC, Font J, Lie JT, Burcoglu A, Lim K, Munoz-Rodriguez FJ, Levy RA, Boue F et al (1998) Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. Medicine (Baltimore) 77:195–207

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Mehmet Sayarlioglu.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Sayarlioglu, M., Topcu, N., Harman, M. et al. A case of antiphospholipid syndrome presenting with pulmonary truncus and main pulmonary artery thrombosis. Rheumatol Int 25, 65–68 (2005). https://doi.org/10.1007/s00296-004-0458-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00296-004-0458-9

Keywords

Navigation